ABSTRACT
The frequency of anatomical variation of the bile duct system is relatively common. The constitution of a normal biliary confluence by union of the right and left hepatic ducts is reported in only 57% (Couinaud 1957) to 72% (Healy & Schroy 1953) of cases. While many of these variations have little or no clinical importance, some cases cause symptoms and signs, or may lead to incorrect diagnosis and inappropriate management of biliary disorder. We report a case of rare extrahepatic bile duct anomaly in a 60-year-old man. His chief complaint was intermittent right upper quadrant pain for six months.There was a low union of both extrahepatic ducts and the cystic duct. The left extrahepatic duct fused with the right extrahepatic duct anteriorly and the long cystic duct ran alongside and parallel with the right and left extrahepatic duct before joining them. Distal CBD cancer was also combined.